Neurological Disorders: Craniopharyngioma

Introduction

Craniopharyngioma is a benign (non-cancerous) type of brain tumor that is extra axial in nature. It is predominantly a tumor during the childhood/younger age however there is another peak later in life.

Extra axial means the tumor originates outside the brain, but may push the neighboring brain tissue or infiltrate into it.

It originates from the remnants of rathke pouch. From this pouch the anterior part of the pituitary gland develops.

It is a slow growing benign tumor however because of its location abutting brain tissue, hypothalamus, pituitary gland, optic nerve (nerve of vision, the 1st cranial nerve) etc this tumor can cause significant morbidity.

Clinical Manifestations

The following manifestations are usual;

• Headache
• Visual field defects
• Endocrine abnormalities etc

Diagnosis

MRI of the brain with & without contrast with a good focus towards the pituitary area is the investigation of choice. A CT head is fine too but MRI has some edge. Presence of calcification is an important finding and actually a CT will detect calcium more easily than MRI so CT is definitely useful but MRI gives more detailed picture of the intracranial contents.

A biopsy is needed to confirm the diagnosis.

Treatment

The emphasis is on total excision of the tumor if that is possible. Trans-sphenoidal or sub-frontal surgical approaches are tried. If not possible to totally remove the tumor then post surgical radiation therapy needs to be given. Chemotherapy has some role too. Recurrence of the tumor is not uncommon.

A pediatric and/or adult neurologist along with neuro-surgeons, neuro-oncologists are frequently involved in the care of patients with this tumor.



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